A 51-year-old woman developed small cell lung cancer (SCLC). She was a non-smoker and had interstitial pneumonia associated with systemic sclerosis (SSc). Sixteen additional cases obtained from the literature describing patients with SCLC associated with SSc are reviewed. The majority of patients were women with underlying interstitial pneumonia. In 3 patients who were non-smokers, interstitial pneumonia was complicated and cancer had developed in the peripheral lung field but not in the central lung field. Since SCLC is very rare in non-smokers, these findings suggest a positive association between SCLC and interstitial pneumonia associated with SSc.